The European Respiratory Society released updated guidelines in 2026 for the treatment and management of patients with pulmonary arterial hypertension (PAH). Primary changes include the addition of sotatercept as second-line treatment for intermediate-low to high-risk patients as well as recommendations for risk stratification.
- In 2024, the FDA and EMA approved sotatercept — the first activin signaling pathway inhibitor for treatment of PAH.
- The updated guidelines recommend sotatercept as second-line treatment for intermediate-low to high-risk patients uncontrolled on first-line oral therapies.
- Guidelines emphasize right heart catheterization for risk monitoring and individualized treatment strategies at specialized PAH centers.
- Evidence-based recommendations are supported by randomized controlled trials including HYPERION, PULSAR, STELLAR and ZENITH studies.
The European Respiratory Society (ERS) announced updated clinical practice guidelines for the treatment of pulmonary arterial hypertension (PAH). The revised recommendations reflect important therapeutic advancements and recent research on risk stratification, according to lead author Gabor Kovacs, MD, of the Ludwig Boltzmann Institute for Lung Vascular Research in Graz, Austria.
The ERS presented the new guidelines at its Congress 2026 in Barcelona, Spain, and subsequently published them in the European Respiratory Journal. They replace the 2022 European Society of Cardiology/ERS recommendations, which previously introduced therapeutic approaches targeting three core signaling pathways: endothelin, nitric oxide and prostacyclin.
“However, since then, major advances have expanded the therapeutic arsenal,” said Dr. Kovacs in a news release. “In 2024, sotatercept, the first activin signaling pathway inhibitor of its class, was approved by the FDA and EMA for the treatment of PAH. An update was necessary.”
The addition of sotatercept provides a fourth pathway-targeted therapy that offers benefits to patients with PAH who are already receiving standard-of-care treatment and whose mortality risk is intermediate-low, intermediate-high or high at the time of follow-up, wrote the ERS Task Force. This recommendation is based on evidence from multiple randomized controlled trials, the authors noted, including HYPERION, PULSAR, STELLAR and ZENITH.
The updated guidelines also further address the role of right heart catheterization (RHC) in PAH diagnosis and management. RHC is strongly advised for patients with intermediate-low, intermediate-high or high risk to help monitor therapeutic efficacy and safety.
Due to limited evidence, the task force does not support routine use of sotatercept or RHC in patients with PAH whose risk is low. Rather, it recommends selecting an appropriate treatment strategy on a case-by-case basis and in a PAH specialized treatment center.
The task force developed four patient, intervention, comparator, outcome (PICO) questions as well as a treatment algorithm to guide shared decision-making.
According to the guidelines, oral therapies like phosphodiesterase type 5 inhibitors and endothelin receptor antagonists remain the first-line treatment, with the addition of sotatercept as second-line treatment for those whose disease remains uncontrolled. Other therapeutic options, including prostacyclins or lung transplantation, are available for patients with persistent disease.
“The goal is to bring as many patients as possible to low-risk status, which is associated with a more favorable prognosis and lower mortality,” said David Montani, MD, PhD, a pulmonologist at the French National Referral Center for Pulmonary Hypertension at Bicêtre Hospital in Le Kremlin-Bicêtre, France.
